Endomyocardial fibrosis is a progressive disease of unknown
origin affecting children and young adults. It involves inflow portion
of right and/or left ventricle and apex. It may be associated with
thrombus. Literature regarding right ventricular endomyocardial
fibrosis with thrombus is scarce. Here we report a rare case of right
ventricular endomyocardial fibrosis presenting as ventricular
tachycardia and echocardiographic evidence of apical thrombus.
Interestingly there was no pulmonary involvement or evidence of deep
venous thrombosis. This case also underscores the importance of urgent
echocardiography in diagnosis of obscure cases of ventricular
tachycardia.

Key words: Endomyocardial
Fibrosis; Ventricular Tachycardia

Case Summary

A 36-yr-old female presented to emergency with the complaint of sudden
onset loss of consciousness for 2-3 minutes. She spontaneously regained
her consciousness after this episode. Her unconsciousness was not
associated with trauma, seizures, focal neurological deficits,
confusion, chest pain, palpitation, fever, diarrhea or vomiting. She
had no past history of coronary artery disease, cerebrovascular
accident, seizure disorder, tuberculosis, hypertension or similar
episode of unconsciousness. She had two children, both delivered at
full term. The last child birth was 7 years back. There was no history
of abortions or stillbirth. She denied any intake of oral
contraceptives, or any other medication She was non alcoholic and non
smoker. There was no history of sudden cardiac death or similar disease
in the family. On presentation her pulse was rapid and feeble
with monitor showing heart rate of 300/min, blood pressure of 60 mm of
Hg systolic and respiratory rate of 30/min.

ECG showed evidence of ventricular tachycardia (VT) with an inferior
axis and left bundle branch morphology (Figure
1A) for which she was given 360 J of synchronized DC shock with
subsequent reversion to normal sinus rhythm and stabilization of blood
pressure. The QTc was 0.447 secRepeat ECG showed right bundle branch
block with T wave inversion in leads V2-V6, II, III, aVF with left axis
deviation (Figure 1B). An
urgent echocardiography was performed which showed obliteration of the
right ventricular apex, a thrombus lodged at the apical area,
restrictive flow pattern across the tricuspid valve, enhanced density
of the right intraventricular band and enlarged right atrium that was
suggestive of right ventricular endomyocardial fibrosis with apical
thrombus with normal pulmonary valves and no regional wall motion
abnormality (Figure 2A). The
Doppler TR gradient was 22 mm of Hg. Her baseline serum biochemistry
including thyroid profile, lipid profile and blood sugar was normal.
CPK-MB was not raised and Troponin T was negative. Her hs-CRP
(24.30mg/L), homocysteine (16.46µmol/L), fibrinogen (571mg/dl)
and D dimer (18.79µg/ml) were elevated. Her antiphospholipid IgG
and IgM antibodies; Protein C, functional and Protein S, functional
were within normal range. Contrast enhanced computed tomography of
chest showed no evidence of pulmonary thromboembolism, pulmonary
hypertension or parenchymal involvement. Doppler ultrasound of
bilateral lower limbs and carotid was normal. Based on the above
clinical features and criteria for diagnosis and assessment of severity
of endomyocardial fibrosis [1] (i.e 2 major or 1 major and 2 minor
criteria) a diagnosis of moderate right ventricular endomyocardial
fibrosis was established. These criteria have been validated in a study
on 1063 subjects from rural Mozambique [1]. She was treated with
anticoagulants (warfarin) and amiodarone. She improved on this
treatment. Subsequent echocardiography revealed decrease in size of
right ventricular thrombus (Figure 2B).
Patient is still on regular follow up.

Figure 1

Figure 2

Discussion

Endomyocardial fibrosis is a relatively frequent cause of heart failure
and death in African countries [2]. However, its occurrence in most
parts of India and Asian countries is uncommon [3]. It is characterized
by fibrous endocardial lesions of the inflow of the right or left
ventricle or both and often involves in atrioventricular valves
resulting in regurgitation [4]. The disease affects both sexes equally
and is common in children and young adults. Combined right and left
ventricular disease occurs in about 50% cases with pure left
ventricular involvement occurring in 40% and pure right ventricular
involvement in the remaining 10% population [5]. Pure right ventricular
involvement usually shows extensive fibrous thickening of the inflow
tract and apex with a mass of thrombus [4]. The echocardiographic
criteria for diagnosis include 6 major criteria (endomyocardial plaques
>2 mm, thin (≤1 mm) endomyocardial patches in more than one
area of ventricular wall, obliteration of ventricular apex, thrombi
without severe ventricular dysfunction, right ventricular apical notch
and AV valve dysfunction secondary to valvular adhesion to ventricular
wall) and 7 minor criteria (one ventricular wall affected by
endomyocardial patches, restrictive flow across atriventricular
valves, diastolic opening of pulmonary valve, diffuse thickening of the
anterior mitral leaflet, enlarged atrium with normal-size ventricle,
M-movement of the interventricular septum and flat posterior wall, and
increased density of the moderator or other intraventricular bands). To
establish the diagnosis of endomyocardial fibrosis 2 major or 1 major
and 2 minor criteria are needed [1]. Our patient clearly had 2 major
criteria including obliteration of right ventricular apex and right
ventricular thrombus. Three minor criteria including restrictive flow
pattern across the tricuspid valve, enhanced density of the right
intraventricular band and enlarged right atrium were also seen. The
severity score was 13 indicative of moderate disease.

Besides endomyocardial fibrosis right ventricular thrombus has also
been seen in a variety of other clinical conditions such as
hypercoagulable state, i.e. protein C and S deficiency [6], Behcet's
disease [7], Loffler endocarditis [8], amyloidotic heart disease [9]
etc. However in our case the above clinical conditions were ruled out
by appropriate investigations as cited above.

The mainstay of treatment of endomyocardial fibrosis is surgical
excision of fibrous endocardium and replacement of damaged valves [10].
When surgery is contraindicated or denied by the patient, medical
therapy should be opted. If right or left ventricular failure sets in,
digitalis and diuretics should be added [11,12]. In case of intramural
thrombus, anticoagulation should be initiated if no contraindication
exists. Our patient presented for the first time with ventricular
tachycardia which was later found to be associated with right
ventricular endomyocardial fibrosis and apical thrombus. She was
started on anticoagulants and anti-arrhythmics after which she became
asymptomatic. The present case illustrates the importance of urgent
echo in diagnosing obscure causes of ventricular tachycardia presenting
in emergency.